------------------------------------------------------------------------------
TITLE: ENT APPROACH TO ORBITAL TUMORS
SOURCE: Dept. of Otolaryngology, UTMB, Grand Rounds
DATE: 5 November 1991
RESIDENT PHYSICIAN: Lane F. Smith, M.D.
FACULTY: Karen H. Calhoun, M.D.
DATABASE ADMINISTRATOR: Melinda McCracken, M.S.
------------------------------------------------------------------------------
"This material was prepared by resident physicians in partial fulfillment of
educational requirements established for the Postgraduate Training Program of
the UTMB Department of Otolaryngology/Head and Neck Surgery and was not
intended for clinical use in its present form. It was prepared for the purpose
of stimulating group discussion in a conference setting. No warranties, either
express or implied, are made with respect to its accuracy, completeness, or
timeliness. The material does not necessarily reflect the current or past
opinions of members of the UTMB faculty and should not be used for purposes of
diagnosis or treatment without consulting appropriate literature sources and
informed professional opinion."
I. IMPORTANT ANATOMY
A. In General
1. Orbit is a quadrilateral pyramidal cavity composed of seven bones.
2. The posteromedial orbital floor is the weakest portion.
3. There are no lymphatics in the orbit.
B. Inferior Orbital Wall
1. Composed of orbital plate of maxilla (largest portion) zygomatic bone
anterolaterally and orbital plate of the palatine bone.
2. The orbital floor is separated from the lateral wall by the inferior
orbital fissure, no sharp line of demarcation from medial wall.
3. Canal for infraorbital nerve runs here and weakens this portion of
orbit.
4. Inf. oblique muscle attaches anteromedially here.
C. Medial Orbital Wall
1. Composed of frontal process of maxilla, lacrimal bone, lamina
papyracea of ethmoid, and part of lesser wing of sphenoid.
2. Contains the optic foramen (which is not located at the geometric
apex of the orbit) and foramina for ant. and post. ethmoid arteries.
3. Anteriorly, lacrimal fossa which contains the lacrimal sac.
4. Distance between post. ethmoid foramen varies from 4mm to 7mm.
5. The optic foramen contains optic n. and ophthalmic artery.
6. The posteromedial orbital floor is the weakest portion of the orbit.
D. Lateral Orbital Wall
1. Composed of frontal process of the zygoma, greater wing of sphenoid
lateral to optic foramen.
2. Whitnall's tubercle, a bony prominence located just deep to vein and
just above midpoint of lat. wall, lateral canthal ligament attaches
here.
3. Inf. orbital fissure contains:
a. Path of communication between orbit and infratemporal and
pterygopalatine fossae.
b. Maxillary division of trigeminal n. (V2).
c. Infraorbital artery.
d. Branches of sphenopalatine ganglion.
4. Superior orbital fissure makes boundary between lateral orbital
wall and superior wall.
a. Posteriorly communicates with cavernous sinus and middle cranial fossa.
b. Transmits:
(1) Oculomotor n. (CN III)
(2) Trochlear n. (CN IV)
(3) Abducens n. (CN VI)
(4) Terminal branches of ophthalmic n. (CN V1)
(5) Superior ophthalmic vein.
E. Superior Orbital Wall
1. Composed of the orbital plate of the frontal bone, with a small
contribution from the lessor wing sphenoid.
2. Anterolaterally fossa for lacrimal gland.
3. Medially 5mm deep to rim, the trochlea inserts.
F. Orbital Fasciae
1. Periorbita.
a. Is the periosteum lining the bony wall and is a continuation of
the dura mater from the optic foramen and sup. orbital fissure.
b. Superiorly forms superior oblique tendon.
c. Inferomedially splits to enclose the lacrimal sac.
d. Septa divides orbital fat into lobules.
e. Subperiosteal space: surgical plane between orbital walls and
periosteum.
2. Orbital septum or palpebral fascia.
a. Covers the entrance of the orbit and is a continuation of the
periorbita at the rim.
b. On upper lid unites with levator aponeurosis.
c. On lower lid fuses with tarsus and inf. rectus.
d. Attaches to posterior aspect of levator aponeurosis.
3. Bulbar fascia or tenons capsule.
a. A fibrous sheath surrounding entire globe except cornea.
b. Separates eye from orbital contents.
c. Anteriorly perforated by tendons of rectus muscles.
d. Surgical space between bulbar fascia and globe is the episcleral
space.
4. Muscular fascia.
a. Formed by the fibrous sheathes of the extraocular muscles.
b. Thickening of this process from the medial and lateral recti forms
the medial and lateral canthal tendons.
c. Canthal tendons and fascia from medial lat and inf. recti blend
together to form a fibrous sling supporting the globe known as the
suspensory ligament of Lockwood.
G. Orbital Vessels
1. Main blood supply is ophthalmic artery, a branch of internal carotid.
2. The ant. and post. ethmoidal branch of the ophthalmic and exit medial
orbital wall through ant. and post. ethmoidal foramina.
3. Lower part of orbit supplied by infraorbital artery, a branch of the
IMA which a branch of the ECA.
4. Venous drainage of the orbit is valveless with numerous
interconnections leading to easy spread to the cavernous sinus.
H. Extraocular Muscles
1. Arise from a common tendinous ring, the annulus of Zin which is at
the apex encircles the sup. orbital fissure and optic foramen.
2. As mentioned, muscular fasciae fuses with the bulbar fascia
anteriorly.
3. Fasciae and muscles form the muscular cone which separates central
from peripheral surgical space.
I. Four Surgical Spaces
1. Subperiosteal space; lies between periosteum and bony orbit.
2. Episcleral space; lies between bulbar fascia and globe (sclera).
3. Central surgical space.
a. Lies under muscular cone. (See above.)
b. Contains:
(1) Optic n.
(2) CN III
(3) CN V (Nasociliary branch.)
(4) CN VI
(5) Ophthalmic artery and its major branches.
(6) Ophthalmic veins.
4. Peripheral surgical space.
a. Lies outside of muscular cone.
b. Contains orbital fat.
c. Contains CN IV.
J. Lacrimal System
1. Secretory system consists of two major types of glands; basic and
reflex secretors.
2. Basic secretors: consists of numerous small goblet cells in
conjunctiva, tarsal meibomian glands and accessory lacrimal glands
of the subconjunctiva of the upper lid.
3. Reflex secretors consists mainly of the lacrimal gland which is
located in the lacrimal fossa in the lateral orbit superiorly and
anteriorly. This gland contains two to six excretory ducts.
4. Excretory system.
a. Tears from the basic and reflex secretions are pumped by eyelid
action medially.
b. There is a lacrimal punctum in the upper and lower lid. (The
punctum is the opening of the canaliculus.)
c. Both the superior and inferior canaliculus have a 2mm vertical
component which makes a 90 degree turn to a horizontal component
8mm in length.
d. The upper and lower canaliculus join to form a canaliculus which
empties into lacrimal sac just post. and sup. to the center of
its lateral wall.
e. The lacrimal sac empties into the nasolacrimal duct which opens
in the inferior nasal meatus.
K. Eyelids
1. Anterior lamella: consists of skin and orbicularis muscle.
2. Posterior lamella consist of tarsus and conjunctiva.
II. EVALUATION OF ORBITAL TUMORS
A. A good history of onset of lesion, diplopia, visual disturbance,
loss of visual acuity, pain, photophobia, acute or chronic onset,
etc. is necessary.
B. Extensive physical examination of the orbital area, periorbital area,
paranasal sinuses, visual acuity, and cranial nerves.
C. Radiographic studies as indicated.
1. CT scanning is the best radiographic modality for extraocular orbit.
2. Axial and coronal scans of orbit and paranasal sinuses are essential
for evaluating extent of the lesion and planning surgery.
D. Unilateral Proptosis
1. Graves ophthalmopathy is most common cause in adults, cellulitis
(secondary to paranasal sinusitis) in children.
2. Pseudotumor second most common cause in both pediatric and adults.
3. Next most common cause in adults is paranasal sinus tumors.
(See table 1.)
4. Often history, physical, plain radiographs and laboratory tests
establish the diagnosis in approximately 50% of the patients.
With CT scanning the yield is even higher.
TABLE 1. ORBITAL TUMORS (most to least frequent causes not including orbital
pseudo tumor)
Adults Pediatric
1. Paranasal sinus tumors 1. Dermoids and epidermoids
2. Metastases (often breast/lung) 2. Capillary hemangiomas
3. Cavernous hemangiomas 3. Lymphangiomas
4. Lacrimal gland tumors 4. Rhabdomyosarcomas
5. Lymphomas 5. Neurofibrands & optic gliomas
6. Meningiomas 6. Leukemias and lymphomas
7. Dermoids and epidermoids 7. Metastases (often neuroblastomas)
E. Initial Laboratory Tests
1. CBC: R/O infection.
2. ESR: R/O inflammatory causes.
3. Thyroid function tests: R/O Grave's Disease.
4. Calicum: R/O hyperparathyroidism and brown tumors.
F. Fine Needle Aspiration
1. Very useful when a tumor can be palpated.
2. Should not be used on highly vascular lesions, such as lesions that
enhance significantly on CT scanning. (Thus FNA of the orbit should
probably only be used after contrast enhanced CT scans have been
obtained to R/O vascular lesion.)
3. FNA mostly useful for more anterior lesions although some posterior
tumors adjacent to bony walls may be reached via a
radiographically-guided needle (fluoroscopy or ultrasound).
G. Biopsy
1. Used wherever other modalities (CT, FNA, etc.) have failed to
yield a diagnosis and prior to any radical orbital surgery.
2. Radical orbital surgery differed until permanent sections are back.
Pretreat biopsies must always be performed prior to radical surgery.
3. If the lesion involves paranasal sinuses, biopsy transnasally or
through a paranasal sinus approach (endoscopically, etc.) is
usually easier and preferable to biopsy through the orbit.
4. If lesion is small and easily accessible excisional biopsy is
preferred to incisional biopsy.
H. In general a good history, physical, laboratory tests and plain
fibrous should make the diagnoses in 50% of patients.
III. RADIOGRAPHIC EXAMINATION OF THE ORBIT
A. Plain films: Caldwell, Waters, lateral, optic foramen SMV.
B. CT Scan
1. Best single radiographic modality for evaluating orbital neoplasms.
2. Can sometimes characterize tissue type of neoplastic process.
3. Can delineate extent of neoplastic process and bony destruction.
4. Optic n. sheath meningioma from optic n. glioma. Glioma causes
fusiform enlargement isodense with nerve, whereas meningioma
cylindrically hyperdense giving "railroad track" sign on optic n.
5. Neurofibromas often assoc. with congenital absence of greater and
lesser sphenoid wings, CT shows hemogenous mass of moderate density.
6. Cavernous hemangiomas show up well with contrast as well defined
mass in superolateral muscle cone without apical nerve or muscle
involvement.
7. Lymphangiomas rarely enhance with contrast and are usually outside
the muscular cone.
8. Dermoids and teratomas may appear anywhere in the orbit as
circumscribed variegated lesions with punctate calcification.
9. Embryonal rhabdomyosarcomas, fibrohistiocystomas and lymphomas show
up as average density lesions with moderate enhancement. Lymphomas
are usually outside of the muscular cone.
10. Malignant tumors tend to be poorly defined with destructive bony
changes.
C. Ultrasound
1. Good for solid vs. cystic lesions, hemangiomas and orbital hemorrhage.
2. Modality of choice for intraocular evaluation when opacification
of the lens is present and precludes direct inspection of the
posterior globe.
3. Can clearly distinguish neoplastic invasion from inflammation.
4. A good adjunct to CT scan.
D. Angiography
1. Good for evaluation of vascular masses.
2. Useful for suprasellar masses.
3. Useful for preoperative embolization of vascular tumor (eg.
prior to removal of meningioma).
E. MRI
1. Superior to CT scan in demonstration intracranial extension of mass
lesions.
2. Contraindicated in the evaluation of orbits suspected to harbor
metallic foreign bodies.
IV. PEDIATRIC ORBITAL TUMORS
A. Often requires a multidisciplinary approach involving the
pediatrician, ophthalmologist, oculoplastic surgeon,
otolaryngologist, neurosurgeon and oncologist.
B. Congenital Developmental Cysts
1. Dermoid and epidermoid cysts.
a. Present in preschool children as painless, elevated modules seen
along the superotemporal orbital rim (can be deep or
superonasally).
b. Posterior cysts can cause proptosis.
c. If cyst wall contains adnexal structures (eg. hair, sweat glands,
etc.), called dermoid; if not, epidermoid cyst.
d. Can rarely cause local bony erosion due to pressure.
e. CT scan show low-density lucent lesion.
f. Treatment is complete cyst removal (surgical technique discussed later).
2. Teratoma
a. A congenital rapidly growing tumor present at birth.
b. Present with dramatic exophthalmos.
c. By definition contain all 3 germ layers.
d. Benign tumor but can be massive and extend intracranially.
e. Treatment requires combined neurosurgical and orbital approach
performed soon after birth. Attempt to spare the globe.
C. Vascular Tumors
1. Capillary hemangiomas. (See grand rounds 1989 Vascular Tumors by
Lane Smith.)
a. Reddish to bluish appearance usually present at birth or shortly
thereafter.
b. Usually involve upper eyelids.
c. Can grow rapidly and occlude eye causing amblyopia in as short a
time as two weeks.
d. Usually these lesions will spontaneously involute by age 18 to 36
months.
e. If causing amblyopia, severe cosmetic deformity, or frequent
bleeding can use intralesional steroid injections or surgery
for treatment.
2. Lymphangioma
a. Appear in preschool years.
b. Soft bluish masses usually in the superonasal quadrant.
c. Treatment requires complete surgical removal with sparing of vital
structures.
D. Rhabdomyosarcoma
1. Most common childhood primary malignancy of the orbit.
2. Presents with rapidly evolving exophthalmos in young children
(ages 5-10).
3. Most commonly involves superior orbit.
4. CT shows poorly defined homogenous mass.
5. An incisional biopsy through an anterior approach is used for
diagnosis.
6. Treatment consists of combination radiotherapy and chemotherapy.
7. Exenteration is reserved for rare radioresistant and recurrent tumor.
E. Glioma
1. Present in preschool children with loss of vision proptosis,
papilledema, optic atrophy and strabismus.
2. 25-50% have systemic neurofibromatosis.
3. CT scan shows fusiform enlargement of the optic nerve.
4. Treatment varies from watchful waiting (if vision good, etc.) to
removal of orbital contents.
F. Fibro-osseus Tumors
1. A group of lesions characterized by replacement of normal bony
architecture with tissue composed of varying amounts of collagen
fibroblasts and osteoid and giant cells in differing patterns.
2. Fibrous dysplasia.
a. Most frequent of the fibro-osseous tumors. Two types exist
polyostotic and monostotic fibrous dysphasia.
b. Occurs almost exclusively in children during the first two
decades of life.
c. Polyostotic fibrous dysplasia (Albright's Syndrome).
(1) Multiple bone involvement.
(2) Abnormal skin pigmentation.
(3) Precocious puberty.
(4) Usually spares the orbit.
d. Monostotic fibrous dysplasia.
(1) Occurs most often in face.
(2) Frequently involves the orbit
(3) Often involves maxilla, sphenoid or frontal bone.
e. Radiographic appearance shows a sclerotic lesion with ground glass
appearance.
f. Biopsy necessary to confirm the diagnosis.
g. These often grow rapidly in early life and then stabilize after
puberty.
h. Conservative surgical excision or sculpting of lesion for lesions
causing symptoms.
i. Radiation therapy contraindicated.
3. Ossifying fibroma.
a. Similar to fibrous dysplasia but characterized by a more aggressive
growth pattern.
b. Radiographic appearance more demarcated from normal bone than fibrous
dysplasia.
c. Complete surgical excision is treatment of. Because of the sharp
demarcation from normal bone this is easier to do than with ossifying
fibroma.
4. Osteomas.
a. Uncommon benign tumors of bone.
b. Radiologically well-circumscribed extremely dense masses.
c. Surgical excision is the treatment of choice.
5. Giant-cell lesions. Include giant-cell reparative granuloma, true
giant-cell tumor (rarely occurs in children) and "brown" tumor of
hyperparathyroidism.
G. Metastatic Orbital Tumors.
1. In pediatric age group most common mets are neuroblastoma, Ewing's
Sarcoma, leukemia, medulloblastoma and Wilm's Tumor.
2. Neuroblastoma.
a. Presents with ecchymotic bilateral or unilateral proptosis.
b. Metastases are usually to lateral orbit with bony destruction of
lateral orbital wall.
3. Ewing's Sarcoma presents with abrupt hemorrhagic exophthalmos
usually in second decade.
V. TUMORS OF THE PARANASAL SINUSES, NASAL CAVITY AND NASOPHARYNX INVOLVING THE
ORBIT
A. Paranasal sinus tumors (cancer, tumors and mucoceoles) frequently
extend to involve the orbit.
1. The route of spread is not thought to be secondary to lymphatics as
there is no lymphatic drainage of the orbit.
2. Direct extension through the preformed pathways through anterior and
posterior ethmoid foramina.
3. Tumors from the nasal cavity can extend into the orbit via the nasal
lacrimal duct.
4. The infraorbital canal and inferior orbital fissure sense as a direct
route for cancer invasion from the maxillary liners.
5. Dehiscence of the lamina papracea or lateral wall of sphenoid sinus
allow spread from these areas to orbit.
6. The frontoethmoid, maxilloethmoid, and zygomaticomaxillary suture
lines are also potential routes of spread from paranasal sinuses to
orbit.
7. Periosteum of orbit forms a barrier to tumor spread. Benign tumors
tend to push it aside, malignant tumors invade it.
B. Carcinomas of the paranasal sinus are extremely lethal and rare,
comprising only 0.2% to 0.8% of all carcinomas. These are the most
common tumors to secondarily invade the orbit. Maxillary sinus is
most common sinus origin to invade the orbit.
C. 40-60% of all sinus carcinomas eventually cause enough orbital
involvement to necessitate orbital exenteration.
D. In 70% of tumors involving the orbit bone destruction is present
on CT scan.
E. Clinical Manifestations
1. The majority of cases are detected late, as early on patient are
often asymptomatic.
2. Symptoms of orbital extension include ocular pain, visual alteration,
diplopia, and photophobia.
3. Physical findings of orbital invasion include increased intraocular
pressure, epiphora, ptosis, proptosis, ophthalmoplegia and optic
atrophy.
TABLE 2. NONOSSEOUS TUMORS ORIGINATING IN THE SINUS, NOSE AND OROPHARYNX THAT
EXTEND SECONDARILY INTO THE ORBIT
Mucocele primary or secondary to tumor
Squamous cell carcinoma
Inverting papilloma
Mucoepidermoid carcinoma
Adenocarcinoma
Adenoidcystic carcinoma
Malignant mixed tumor
Angiofibroma
Lymphoepithelioma
Esthesioneuroblastoma
Cholesteatoma
Ameloblastoma
Melanoma of the sinus
F. Radiographic Studies: CT scan best study. (See aforementioned
section on CT scanning and other radiologic studies.)
G. Biopsy of Mass Invading Orbit (See previous section.)
1. As mentioned earlier, biopsy is best obtained transnasally when
possible.
2. Biopsy should be deferred until after neurosurgical consultation
when radiographic studies show communication with cranial cavity,
CSF leak is present or pulsation of mass.
3. If the lesion is highly vascular biopsy should be deferred or
performed in the OR.
4. In general, it is best to wait until after the CT scan and possibly
other radiographic studies prior to biopsy.
H. Benign Neoplasms Invading Orbit
1. Generally do not invade orbital periosteum.
2. True violation of orbital fat or muscular cone infrequent.
3. Tend to push orbital contents aside and cause optic compression.
4. Tend not to have bony invasion although bony evasion via compression
can occur.
5. Include:
a. Inverting papilloma.
b. Osteomas.
c. Other osseous lesions.
(1) Fibrous dysplasia.
(2) Giant-cell granuloma.
(3) Ossifying fibroma.
d. Juvenile angiofibromas.
e. Neuroectodermoid tumors.
(1) Meningiomas.
(2) Schwannomas.
(3) Neurofibromas.
f. Ameloblastoma.
g. Cholesteatomas.
I. Treatment of Benign Lesions Invading the Orbit
1. in general, attempt to spare orbital contents with excision. Orbital
exenteration is indicated with a persistently recurring benign lesion
that threatens to invade cranial cavity or when lesion threatens to
attain unresectability or cause blindness.
2. Tumor may dissected from the periorbita if the periorbita elevates
easily from the bony orbit during resection. If the periosteum has
been penetrated the tumor may be resected with a cuff of fat and
if needed a rectus muscle.
3. Inverting papillomas are usually treated aggressively with a lateral
rhinotomy-medial maxillectomy approach which permits excision of
lateral nasal wall, ethmoid labyrinth and lamina papyracea.
4. Osteomas.
a. Often originate near the frontoethmoid suture.
b. Can be approach via a coronal or Lynch incision.
c. Lesion can be excused via an osteotomy.
d. The floor of the frontal sinus is usually excised, frontal sinus
obliteration is usually not needed, rather an indwelling stent is
placed to create an epithelialized path into the frontal sinus.
5. Angiofibromas usually managed through a transpalatal approach when
small and a lateral rhinotomy approach when larger.
J. Malignant Lesions Invading the orbit
1. Proptosis, invasion through the orbital periosteum muscular cone and
bony walls are common.
2. Squamous cell carcinomas are by far the most common. 90% of all
sinus carcinomas are squamous cell.
3. Concommitant involvement of the maxillary and ethmoid sinuses are
rare except in advanced tumors.
4. Pain occurs secondary to neural (perineural) involvement and is
much more common in malignant than benign lesions. The development
of trismus indicates involvement of the pterygoid fossa.
5. Includes:
a. Squamous cell carcinoma.
b. Mucoepidermoid carcinoma.
c. Adenocarcinoma (important to rule out metastases if this tumor is
found).
d. Adenoid cystic carcinoma.
e. Osteosarcoma.
f. Esthesioneuroblastoma (also called olfactory neuroblastoma).
g. Leiomyosarcoma.
h. Malignant melanoma.
VI. TREATMENT OF PARANASAL SINUS TUMORS, NASAL CAVITY, NASOPHARYNGEAL, AND
OTHER TUMORS INVADING THE ORBIT
A. Combination of radical surgery and radiation therapy, sometimes with
chemotherapy, provides the best cure rates.
B. Even with combination therapy, prognosis with orbital involvement is
extremely poor with 5 year survivals ranging from 10-40%. (Worse for
ethmoid and sphenoid sinus involvement, better prognosis for maxillary
sinus involvement.)
C. Lymphoma, rhabdomyosarcoma and plasmocytoma are usually managed with
non-surgical treatment such as chemo- and radiation therapy.
D. Maxillary sinus tumors with orbital invasion require radical
maxillectomy and orbital exenteration.
E. Malignant tumors of the superior sinuses require craniofacial resection
with simultaneous orbital exenteration. The usual approach involves a
coronal incision with craniotomy resection of the skull base and
transfacial approach either through a rhinotomy or Weber-Ferguson
incision.
F. Orbital Exenteration
1. Gross invasion of the orbit as evidenced by proptosis, limitation of
motion or tumor in the orbital fat or muscular cone indicates the need
for orbital exenteration.
2. Perineural invasion of the optic nerve or involvement of orbital apex
also is a clear indication for orbital exenteration.
3. If there is a question as to whether these structures are involved
frozen section diagnosis can help with the resection.
4. A smooth elevation of the orbital periosteum indicates the orbital
contents can be saved.
5. Controversy exists when there has been pre- operative radiotherapy
and now there is no evidence of orbital involvement. Again,
pre-operative CT scans and frozen section can guide one in these
situations.
6. Previously tumor involvement of the posterior ethmoid cells (with or
without orbital involvement) has been considered an absolute
indication for orbital exenteration. This is now under debate.
Some authors say the orbit can be spared when surgery is guided by
frozen section diagnosis.
7. If only the orbital periosteum is invaded the eye can be saved and
floor of orbit reconstructed with temporalis muscle sling and/or
split thickness skin graft.
G. En Bloc Ethmoidectomy with Medial Maxillectomy (Surgical Procedure)
1. Subperiosteal elevation of medial orbital contents and lacrimal sac,
then retract globe and lacrimal sac laterally.
2. Perform a Caldwell Luc procedure with extensive removal of the
anterior of the maxillary sinus.
3. Next an osteotomy separating the adjacent nasal bone from the nasal
process of the maxilla is performed. Then five major bony cuts are
performed.
4. An osteotome is introduced through the previously opened maxillary
sinus along the inferior medial aspect of the sinus and a cut is made
into the inferior meatus of the nose. (From anterior tip of inferior
turbinate to posterior limits of inf turbinate. This cut ends just
anterior to pterygoid plate.)
5. The next cut is made from floor of medial max. sinus parallel and
just behind the nasolacrimal duct up to superior lacrimal fossa.
6. The third cut is made along the frontoethmoid suture-line beginning
in the lacrimal fossa and extending to just post. to ant. ethmoid
artery.
7. The fourth ant. extends just medial infraorbital nerve, along the
orbital floor diagonally to meet the third cut near the ethmoid
arteries.
8. The final cut is made from the posterior aspect of the floor of
nose up to the posterior tip of the superior turbinate. Any final
attachment of bone is then gently fractured with a to and fro
rocking motion.
9. The nasal bone is returned to its normal position and the medial
canthal ligament is sutured to the adjacent periosteum. The cavity
is lined with split thickness skin graft.
10. Modifications on this technique can be made to include the
cribriform plate, frontal sinus, orbit and nose of the orbital
floor.
H. Reconstruction of the Orbit
1. Can frequently be accomplished by simple placement of a split
thickness skin graft in a sling to hold orbital contents in place
when only floor of orbit has been removed. Some authors state
that if periorbital periosteum intact no reconstruction necessary.
2. If eyelids and conjunctiva are spared after orbital exenteration,
these can be sutured to residual tissue to form a new shallower
socket.
3. Large defects can be repaired with temporalis, galeal, pericranial,
myocutaneous and free flaps.
VII. SKIN CANCER AND THE ORBIT
A. Skin cancers such as squamous cell carcinoma, basel cell carcinoma or
malignant melanoma if allowed to progress untreated can involve the
orbit.
B. Basal cell most common to encroach on orbit.
C. Pathologic diagnosis important prior to any radical surgery as many of
these tumors can be treated with combination therapy and thereby
preserve orbital contents and function.
D. Most often these lesions involve the medial canthus.
E. Conley states the most important feature of management of these
cancers is identification of free margins via Moh's technique or
intraoperative frozen section.
F. The globe can usually be saved although some of the supportive
structures and appendages may have to be resected.
G. Reconstruction is usually with local flaps and/or skin grafts.
VIII. LACRIMAL FOSSA TUMORS AND THEIR MANAGEMENT
A. The superolaterally located lacrimal gland is the sole epithelial
structure in the orbit. (See aforementioned anatomy.)
1. The entire gland can usually be sacrificed with no need for
artificial tears.
B. Tumors here account for 10-15% al all primary orbital tumors
and are equally divided between nonepithelial and epithelial
disorders.
1. Nonepithelial lesions (in order of frequency).
a. Pseudotumor.
b. Benign lymphoid hyperplasia.
c. Lymphoma.
d. Sarcoid.
e. Sjogrens.
2. Epithelial tumors (in order of frequency).
a. Pleomorphic adenoma.
b. Adenoid cystic carcinoma.
c. Adenocarcinoma.
d. Undifferentiated carcinoma.
e. Mucoepidermoid carcinoma.
f. Metastases.
g. Squamous cell carcinoma.
C. Signs and symptoms of lacrimal fossa tumors. (Note: often history
and onset can lead to fairly accurate diagnosis.)
1. Benign mixed tumor.
a. Slow, painless growth over 1 to 2 years.
b. Minimally symptomatic.
c. CT scan shows smooth rounded mass with no bone evasions.
2. Pseudotumor.
a. Painless mass presenting over course of a few weeks to 1-3 months
sometimes faster.
b. Not erythematous, tender or signs of systemic infection (ie. no fevers).
c. Should regress to 50% of size with 2 week trial of antibiotic.
3. Carcinomas.
a. Painful (sometimes painless) fairly rapid growth.
b. CT scan shows irregular margins with bony evasion or invasion.
4. Infections (eg. acute dacryoadenitis).
a. Painful inflamed, erythematous.
b. Progress over to days to weeks.
c. Responds to antibiotics.
D. Diagnosis
1. Suspected infections such as acute dacryoadenitis respond to
antibiotics and need no further work-up (other than possibly CT
scans and cultures).
2. Pseudotumors respond dramatically to trial of steroids; no
histologic diagnosis needed.
3. All other lesions require histologic diagnoses.
a. Fine needle aspiration.
b. Benign mixed tumors require complete excisional biopsy. If a
previous incisional biopsy has been performed, the lesion and the
surgical tract with a cuff of normal tissue must be excised.
c. Carcinomas can undergo an incisional biopsy with permanent sections
prior to orbital exenteration or other radical surgery.
E. Treatment
1. Pseudotumor: steroids.
2. Infections: antibiotics.
3. Benign mixed tumors.
a. Noninvasive lacrimal fossa mass lesions are excised in total without
preliminary biopsy with the expectation of a benign mixed tumor.
b. The periosteum of the lacrimal fossa the main body of the gland and
the conjunctiva around the excretory ducts in the palpebral lobe are
removed in bloc.
c. If a previous incisional biopsy has been made, the entire biopsy tract
with a generous cuff of adjacent tissue is included in the resection.
4. Low-grade mucoepidermoid carcinoma: wide excision of the gland,
adjacent periosteum and levator muscle.
5. Epithelial malignancies.
a. Require orbital exenteration including upper lid and lateral orbital
wall.
b. Extradural removal of the anterolateral orbital roof is necessary.
c. Defect may be repaired with skin graft and/or a temporalis
muscle/pericranial flap.
d. Radiation therapy and chemotherapy may be useful adjuncts to
primary surgical therapy.
e. Prognosis is extremely poor 8-25% 5 year survival.
IX. SURGICAL APPROACHES TO PRIMARY ORBITAL TUMORS
A. Lateral Orbital Tumors
1. Most location for primary orbital tumors.
2. Most common lesion located here is cystic lesions of which
dermoid cyst was most common. (80% of these lesions.)
3. Most often these cystic lesions are located antero- laterally.
4. Frequently inflammatory masses can mask as tumors.
B. Lateral Orbitotomy via Temporal Fossa
1. Because majority of orbital soft tissue is temporal to optic nerve
most primary tumors are accessible by lateral orbitotomy.
2. Especially good for hemangiomas and dermoid/epidermoid lesions.
3. This approach is safe (endangers no important orbital structure).
4. Procedure.
a. A 3 to 3.5cm incision carried straight back from lateral canthus
toward superior attachment of ear. The incision can be extended
up into the brow (wright incision) or can be S-shaped over brow
and temporal region.
b. Undermine just above temporalis fascia.
c. An incision made through temporalis fascia to orbital rim through
periosteum and carried superiorly and inferiorly along orbital rim.
A straight line incision in the direction of the skin incision is
carried posteriorly to the position edge of the zygoma.
d. These superior and inferior flaps are then elevated with a
periosteal elevator and retracted to expose lateral orbital wall.
e. Elevate the lateral canthal tendon from Whitmalls tubercle.
f. A Stryker saw is used to make incisions that are parallel and
slightly converging with lines of the inferior and superior
orbital margins.
g. This segment of bone is then cut-fractured (usually 12mm
posterior to rim). A further 10-12mm of bone can be removed
with drill or rongeurs until the anterior wall of middle cranial
fossa is reached. (Thereby exposing the entire post-lateral orbit.)
h. Place traction suture around lat. rectus and rotate globe.
i. Incise periorbita parallel to lateral rectus over palpable tumor.
j. Remove tumor if not adherent to globe, optic nerve or extraocular
muscles.
k. Reapproximate periorbita attach lat. canthal tendon (via suture
passed through drill hole in that area). Stabilize rim with 28
gauge wires or plates.
l. Place drain in peripheral surgical space and apply a light
pressure dressing over temporal fossa and lids.
C. Surgical Approaches for Lacrimal Gland Masses
1. The lateral orbitotomy approach.
a. Will give access to most lacrimal fossa masses.
b. The superior osteotomy is placed more superiorly; 1cm above the
frontozygomatic suture.
c. If frontal sinus is extensively pneumatized, the superior osteotomy
may enter its lateral portion. An otolaryngologist may need remove
frontal sinus mucosa and perform sinus obliteration if frontal
sinus is diseased.
2. Biopsy of lacrimal fossa mass direct approach.
a. Brow incision can be used.
b. Dissect down directly orbicularis muscle and orbital septum
staying in extraperiosteal plane.
c. Good for incisional biopsy. Not real useful for excisional biopsy.
Use lateral orbitotomy.
D. Medial Orbital Tumors
1. Primary orbital tumors are infrequently located nasal to the optic
nerve. Less than 30% of orbital tumors occur nasal to the optic
nerve.
2. The most frequent diseases in this region are due to direct extension
from the paranasal sinuses.
a. Infections.
b. Mucoceoles.
c. Carcinomas.
d. Benign neoplasms.
3. Of primary orbital tumors that do occur nasal to optic nerve;
peripheral nerve tumors such as meningiomas and gliomas are common.
4. Symptoms usually occur earlier than lateral orbital tumors because
less room to grow which causes early displacement of the globe.
5. The majority of tumors in the nasal orbit are medial to the
extraocular muscles.
E. Surgical Access to Medial Orbital Tumors and Medial Orbitotomy
1. The majority of these tumors can be removed through a Lynch incision
(since they lie medial to the muscular cone).
a. When needed an external ethmoidectomy and/or removal of the orbital
floor medial to the infraorbital nerve can also be performed
through the Lynch incision.
b. The medial and inferomedial spaces can be connected by dividing
the medial canthal tendon, mobilizing the lacrimal sac and
dividing it at the junction with the proximal duct.
c. Medial rectus muscle traction aids in globe rotation thereby
exposing entire medial orbit.
d. This can be performed in combination with the medial maxillectomy.
(See previously mentioned surgical procedure.)
e. Reattach tendon, fracture middle turbinate laterally if the medial
orbital wall has been resected and stabilize nasal pack for 5 days.
2. Dual surgical approach.
a. Used for large tumors or when tumors are located in the central
surgical cone.
b. Perform lateral orbitotomy to allow temporal displacement of globe.
c. Perform external ethmoidectomy and/or removal of the orbital floor
via Lynch excision or medial maxillectomy using lateral rhinotomy
or Weber Ferguson incision.
d. Divide medial canthal tendon, mobilize lacrimal sac laterally and
place medial rectus traction suture.
e. Remove tumor with margins.
f. Collapse ethmoid space by outfracturing the middle turbinate,
stabilize with intranasal pack for 5 days.
g. Reattach canthal tendon, perform a dacryocystorhinostomy.
F. Inferior Orbitotomy
1. For tumors limited to anterior aspect of orbital floor (most
commonly lymphomas and mets).
2. First perform a lateral canthotomy.
3. Retract lower lid inferiorly and incise the conjunctiva in the
inferior fornix directly over the orbital rim.
4. Place traction in bulbar conjunctiva and rotate the globe superiorly.
5. Place malleable under globe for protection.
6. A subconjunctival mass may be exposed through orbital fat, otherwise
dissect in subperiosteal plane to reach mass.
7. The conjunctiva is reapposed with 6-10 chromic.
8. Reattach lateral canthal tendon and close skin.
G. Transantral Decompression
1. Can be used to reach large or posterior orbital floor tumors.
2. Can decompress orbit in unresectable disease to reduce pain or
prolong lesion.
3. Incise periorbital directly over the mass.
4. By combining this with other midface exposures, extensive benign
skull base neoplasms may be resected without sacrifice of vision.
H. Superior Orbitotomy
1. Used to biopsy anterosuperior orbital tumor, lacrimal sac tumors
(see aforementioned section) and excise smaller tumors.
2. Mobilize lacrimal sac from its fossa, mobilize the trochlea.
3. Bipolar cautery of supraorbital vessels.
4. Identify and protect supraorbital neurovascular bundle at notch.
5. Incise periosteum over mass.
6. Control orbital roof bleeders with bone wax.
7. Bluntly separate levator palpebral superior rectus and oblique until
lesion exposed.
8. Dissect mass free if not adherent to other structures. If other
structures involved send incisional biopsy.
9. This procedure can be combined with medial or lateral orbitotomy.
I. Transfrontal or Transcranial Approach to Orbital Tumor
1. This approach is mandatory for lesions which involve both
intraorbital and intracranial compartments.
2. Also used for tumors at the orbital apex in the central surgical
cone and optic canal which cannot be reached by medial or lateral
orbitotomy approaches.
a. The posterior 15mm of the lateral orbital wall encompasses the
superior fissure and cannot be removed with a lateral orbitotomy.
b. Meningiomas, neurofibromas, schwannomas, and tumors of the optic
nerve are often best treated with this approach.
3. A bicoronal flap is raised.
4. The posterior table of the frontal bone is removed and dura is
reflected superiorly.
5. The mucosa of the frontal sinus is removed and frontal duct is
obliterated (optional).
6. The orbital roof and periorbita are then opened.
7. The frontal nerve and artery, trochlear nerve and levator superior
rectus complex are retracted medially or laterally (depending on
location of the tumor).
8. The periorbita is closed.
9. Alloplastic materials, fascia lata or temporalis/pericranial
flaps are placed over the orbit to prevent pulsating exophthalmos.
J. Orbital Exenteration
1. Can remove entire contents of the orbit with or without the eyelids.
2. Usually performed for malignant orbital tumors.
a. Sclerosing basal cell carcinoma.
b. Squamous cell carcinoma.
c. Choroidal melanoma, extrascleral spread.
d. Sarcomas.
e. Lacrimal gland carcinomas.
3. Procedure.
a. 360 degree incision around the orbit 2-3mm outside the rim
through the skin and subcutaneous tissues down to the periosteum.
b. Elevate the periosteum.
c. Release the medial and lateral canthal tendons and trochlea.
d. Remove the lacrimal sac after dividing the nasolacrimal duct,
ablate by cautery.
e. At the level of the superior orbital fissure/optic canal using a
large pair of mayo scissors to divide the orbital contents from
the apex.
f. Hemostasis achieved with monopolar cautery and pressure.
g. Orbit is inspected for tumor extension, remove the sinus or orbital
bones if required.
h. If lids to be retained, the initial incision is made at the gray
line, resecting tissue posterior to the orbicularis oculi muscle.
i. Continue dissection posteriorly until periorbita reached, dissection
completed as mentioned.
j. Defect is filled with vaseline gauze or antibiotic soaked material,
8-14 weeks required before granulation process is completed with
epithelialization of the orbit.
k. Can also line the orbit with a split thickness skin graft.
l. Refer for prosthesis fitting when healed.
------------------------------------------------------------------------------
BIBLIOGRAPHY
1. Sessions RB, Larson DL. En bloc ethmoidectomy and medial maxillectomy.
Arch Otolaryngol 103:195-202, 1977.
2. Kennerdell JS, Maroon JC, Malton ML. Surgical approaches to orbital
tumors. Clinics in Plastic Surgery vol. 15, No. 2, April 1988:273-81.
3. Stasior OG. Evaluation of the lacrimal System for Cosmetic Surgeons.
Arch Otolaryngol 1982;108:495-97.
4. Byers RM, Berkeley RG, et. al. Combined therapeutic approach to
malignant lacrimal gland tumors. Am Journ of Ophthamology vol.
79, No. 1:53-55.
5. Leone CR. Surgical approach to the medial retrobulbar space.
Am Jour of Ophthamology vol. 96, No.1:1-5, 1983.
6. Conley J. The risk to the orbit in head and neck cancer.
Laryngoscope 95; May 1985:515-23.
7. Osguthorpe JD, Saunders RA, et. al. Evaluation of and access to
posterior orbital tumors. Laryngoscope 93;June 1983:766-71.
8. Thawley, Panje, Batsakis, Lindberg eds. Comprehensive management of
head and neck tumors vol. 1., Chapt. 21 controversy in the management
of tumors of the nasal cavity and paranasal sinus. W.B. Saunders
Company, Philadelphia; 1987:445-458.
9. Shields T. Basic principals of management in diagnosis and
management of orbital tumors; W.B. Saunders, Philadelphia, 1989.
10. Osguthorpe JD, Weisman RA, eds. Orbital surgery for head and neck
surgeons. Otolaryngol Clin North Amer, vol 21, No. 1;Feb 1988.
11. Osguthorpe JD, Weisman RA, Tapert MJ. Management of Lacrimal fossa
masses. Arch Otolaryngol Head Neck Surg 1986;112:164-67.
12. Shields JA, Bakewell B, et. al. Classification and Incidence of
Space- Occupying Lesions of the Orbit. Arch Ophthalmol vol.102;Nov.
1984:1606-11.
13. Larson DL, Christ JE, Jesse RH. Preservation of the orbital contents
in cancer of the maxillary sinus. Arch Otolaryngol 1982;108:370-72.
14. Krause CJ, Baker SR. Extended transantral approach to pterygomaxillary
tumors. Ann otol Rhinol Laryngol 91;1982:395-98.
15. Suen JY, Myers EN, eds. Cancer of the head and neck, second edition.
Chapt. 15, Cancer of the nasal cavity and paranasal sinuses. Churchill
Livingstone, New York; 1989.
16. Johns ME, Kaplan MJ, et. al. Supraorbital rim approach to the
anterior skull base. Laryngoscope 94;Sept. 1984:1137-39.
17. Waltman, SR, et. al. eds. Surgery of the eye; Vol. 2. Churchill
Livingstone, New York; 1988.
----------------------------------END------------------------------------------